Jánosi András, Vágó Hajnalka, Hubay Márta
Fővárosi Önkormányzat Szent János Kórháza és Észak-budai Egyesített Kórházai III, Belgyógyászat-Kardiológia, Diósárok út 1-3, Budapest.
Orv Hetil. 2010 Dec 26;151(52):2145-9. doi: 10.1556/OH.2010.28995.
The authors summarize the present knowledge on arrhythmogenic right ventricular cardiomyopathy/dysplasia. Limited data are available about natural history of asymptomatic patients with arrhythmogenic right ventricle cardiomyopathy/dysplasia, who have a ventricular tachycardia during exercise test. A 25-year old female patient was treated with osteosynthesis because of ankle injury. Cardiology consultation was performed because of an abnormal ECG. Physical examination was normal. ECG showed a normal sinus rhythm, left axis deviation, negative T waves in leads II, III, aVF and V2-V6. Chest X-ray and laboratory findings were normal. Echocardiography showed normal left ventricular ejection fraction along with inferior akinesis and dilated right ventricle. Bicycle exercise test revealed a good exercise tolerability (9 MET), and after sporadic ventricular extra systoles ventricular tachycardia developed lasting for 3 minutes, which spontaneously stopped after aborting the test and performed abdominal strain. MRI was performed which has shown normal left ventricular size, wall motion and ejection fraction and depressed right ventricle function (ejection fraction 31.6%) enlarged right ventricular end-systolic and diastolic volumes, hypo-akinetic regions without aneurysm and bulging. No contrast enhancement was seen in the thin right ventricular wall. According to abnormal ECG and MRI findings arrhythmogenic right ventricle cardiomyopathy/dysplasia was diagnosed. No ICD implantation was indicated because the patient was asymptomatic, and no sudden cardiac death occurred in the family. Three month later the patient was found dead. At autopsy the right ventricular chamber was markedly enlarged, with multiple translucent areas of fatty accumulation accompanied with extended myocytes loss. There was a characteristic triangle dysplasia: the inflow, outflow tracts and apical areas. The coronaries were free of atherosclerosis. Mallory's phosphotungstic acid-hematoxilin stain demonstrated the presence of fibrosis within the scattered myocardium.
malignant ventricular arrhythmia provoked by exercise test in an asymptomatic arrhythmogenic right ventricle cardiomyopathy/dysplasia patient with negative family history should be an indication for ICD implantation.
作者总结了关于致心律失常性右室心肌病/发育异常的现有知识。关于运动试验期间出现室性心动过速的无症状致心律失常性右室心肌病/发育异常患者的自然病史，可用数据有限。一名25岁女性患者因脚踝受伤接受了骨固定治疗。因心电图异常进行了心脏科会诊。体格检查正常。心电图显示窦性心律正常，电轴左偏，II、III、aVF及V2 - V6导联T波倒置。胸部X线和实验室检查结果正常。超声心动图显示左室射血分数正常，下壁运动减弱，右室扩大。自行车运动试验显示运动耐量良好（9代谢当量），偶发室性早搏后出现持续3分钟的室性心动过速，试验中止并进行腹部用力后自行终止。进行了MRI检查，结果显示左室大小、壁运动和射血分数正常，右室功能降低（射血分数31.6%），右室收缩末期和舒张末期容积增大，无瘤样扩张和膨出的运动减弱区域。右室薄壁未见对比增强。根据心电图和MRI异常表现，诊断为致心律失常性右室心肌病/发育异常。由于患者无症状且家族中无心脏性猝死发生，未建议植入ICD。三个月后患者被发现死亡。尸检发现右室腔明显扩大，有多个脂肪堆积的半透明区域，伴有广泛的心肌细胞丢失。存在特征性的三角形发育异常：流入道、流出道和心尖区域。冠状动脉无动脉粥样硬化。马洛里磷钨酸苏木精染色显示散在心肌内存在纤维化。
对于家族史阴性的无症状致心律失常性右室心肌病/发育异常患者，运动试验诱发的恶性室性心律失常应作为植入ICD的指征。
